Selective Transgenic Expression of Mutant Ubiquitin in Purkinje Cell Stripes in the Cerebellum

Bert M. Verheijen; Romina J.G. Gentier; Denise J.H.P. Hermes; Fred W. van Leeuwen; David A. Hopkins

doi:10.1007/s12311-016-0838-1

Selective Transgenic Expression of Mutant Ubiquitin in Purkinje Cell Stripes in the Cerebellum

Bert M. Verheijen, Romina J.G. Gentier, Denise J.H.P. Hermes, Fred W. van Leeuwen, David A. Hopkins

Medicine

Medicine

Résultat de recherche: Article › examen par les pairs

1 Citation (Scopus)

Résumé

The ubiquitin-proteasome system (UPS) is one of the major mechanisms for protein breakdown in cells, targeting proteins for degradation by enzymatically conjugating them to ubiquitin molecules. Intracellular accumulation of ubiquitin-B⁺¹ (UBB⁺¹), a frameshift mutant of ubiquitin-B, is indicative of a dysfunctional UPS and has been implicated in several disorders, including neurodegenerative disease. UBB⁺¹-expressing transgenic mice display widespread labeling for UBB⁺¹ in brain and exhibit behavioral deficits. Here, we show that UBB⁺¹ is specifically expressed in a subset of parasagittal stripes of Purkinje cells in the cerebellar cortex of a UBB⁺¹-expressing mouse model. This expression pattern is reminiscent of that of the constitutively expressed Purkinje cell antigen HSP25, a small heat shock protein with neuroprotective properties.

Langue d'origine	English
Pages (de-à)	746-750
Nombre de pages	5
Journal	Cerebellum
Volume	16
Numéro de publication	3
DOI	https://doi.org/10.1007/s12311-016-0838-1
Statut de publication	Published - juin 1 2017

Note bibliographique

Funding Information:
Acknowledgements The authors would like to thank Dr. Richard Hawkes (University of Calgary, Calgary, AB, Canada) for his generous gift of anti-zebrin II antibody and Dr. Carol Armstrong (Mount Royal University, Calgary, AB, Canada) for advice. FWvL received funding from the Internationale Stiching Alzheimer Onderzoek (ISAO, grant # 06502 and 09514), Hersenstichting Nederland (2008.17 and 15F07.48), IPF 2008, and Van Leersum Foundation KNAW 2011. DAH was supported by an ISAO visiting professorship.

Publisher Copyright:
© 2016, The Author(s).

ASJC Scopus Subject Areas

Neurology
Clinical Neurology

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10.1007/s12311-016-0838-1

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abstract = "The ubiquitin-proteasome system (UPS) is one of the major mechanisms for protein breakdown in cells, targeting proteins for degradation by enzymatically conjugating them to ubiquitin molecules. Intracellular accumulation of ubiquitin-B+1 (UBB+1), a frameshift mutant of ubiquitin-B, is indicative of a dysfunctional UPS and has been implicated in several disorders, including neurodegenerative disease. UBB+1-expressing transgenic mice display widespread labeling for UBB+1 in brain and exhibit behavioral deficits. Here, we show that UBB+1 is specifically expressed in a subset of parasagittal stripes of Purkinje cells in the cerebellar cortex of a UBB+1-expressing mouse model. This expression pattern is reminiscent of that of the constitutively expressed Purkinje cell antigen HSP25, a small heat shock protein with neuroprotective properties.",

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AB - The ubiquitin-proteasome system (UPS) is one of the major mechanisms for protein breakdown in cells, targeting proteins for degradation by enzymatically conjugating them to ubiquitin molecules. Intracellular accumulation of ubiquitin-B+1 (UBB+1), a frameshift mutant of ubiquitin-B, is indicative of a dysfunctional UPS and has been implicated in several disorders, including neurodegenerative disease. UBB+1-expressing transgenic mice display widespread labeling for UBB+1 in brain and exhibit behavioral deficits. Here, we show that UBB+1 is specifically expressed in a subset of parasagittal stripes of Purkinje cells in the cerebellar cortex of a UBB+1-expressing mouse model. This expression pattern is reminiscent of that of the constitutively expressed Purkinje cell antigen HSP25, a small heat shock protein with neuroprotective properties.

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Selective Transgenic Expression of Mutant Ubiquitin in Purkinje Cell Stripes in the Cerebellum

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